June this year has flashbacks to 2009.  Despite the long grey winter and spring, this month has improved by the week.  As I write this, in the last days of the month, we are in the throws of a sun-drenched Wimbledon and (can it be true?) the beginning of a drought!

Our annual medical meeting “Ten Topics” is to be held as usual at the start of July, this year on the 1st and 2nd.  Two hundred doctors registered for this wonderful meeting.  International guest speakers.  Catering planned.  Nothing can go wrong?  (Last year was just as hot: the air conditioning in the lecture theatre at St Thomas’ Hospital failed.)

As usual, quite a chunk of the two day meeting will be devoted to Lupus and to Hughes Syndrome, with newer aspects of treatment and ‘hot topics’ including, for example, new data on how anti-phospholipid antibodies might cause thrombosis and miscarriage.  My own talk this year is on an interesting question that is “are there circumstances other than thrombosis where anticoagulation might be beneficial?”.  Today’s ‘patient of the month’ could be one such example.

Patient of the Month

“I failed a job application of Wendy’s”

Mrs P.A., a 40 year old American lady had been diagnosed with Hughes Syndrome following three miscarriages and a possible previous thrombosis, first treated with warfarin but now on aspirin.

At first things had gone well, but more recently she had slowed down, partly because of aches and pains in both hips and possibly more seriously, because of increasing chest discomfort on walking up slopes.

Hip x-rays were normal, but cardiac testing showed slightly impaired blood supply to the heart muscle, despite normal–looking coronary arteries.

Blood tests revealed high anticardiolipin (aCL) levels and, a ‘blast from the past’, a false positive test for syphilis.

It was decided to change treatment to warfarin.  Not only was there an immediate improvement in the chest discomfort (and in the heart tests), but to everyone’s surprise, the hip pains disappeared as well!

What is this patient teaching us?

Firstly the ‘false positive’ test for syphilis.  One of the early serum tests for syphilis (in fact one of the very earliest test in immunology) was named after its inventor, the “Wasserman reaction” or “WR”.  At first, a positive “WR” test was thought to indicate infection with syphilis.  Later it was recognised that “false positive WR” tests could occur in other individuals, mainly lupus patients.  We now know that those unfortunate Lupus patients, including our 40 year old, who as an innocent 16 year old was devastated by being turned down for a job in Wendy’s fast food outlet because of a positive WR test.  In retrospect, of course, she was carrying the aPL antibody.  A close friend of my wife and I, herself a Lupus patient from Cyprus, once told me how she and her husband had once planned a life in America, but were turned down by a (false) positive WR test.

There is another lesson to be learnt from this story.  We have always suspected that in many cases the telltale antibodies appear long before the disease.  In a recent widely reported study from America, a group in Oklahoma traced the records (and blood samples) of US military recruits.  As might be guessed, a number of Lupus patients with previous army records had had positive Lupus tests years (and sometimes decades) prior to clinical symptoms.  The failed “Wendy teenager” might be a similar example.  But even more interesting for the medical sleuths is the improvement not only in the chest pains, but also in the bilateral hip pain.

In previous publication and ‘blogs’ I have attempted to emphasise how important the syndrome might be in the world of cardiology.  We have, for example, seen a number of patients with angina-like pain in whom the coronary main motorways seem clear (sometimes called rather mysteriously “Syndrome X”.  Presumably the ‘stocky blood’ is affecting the smaller vessels in the heart, the highways and byways rather than the coronaries, which more usually affected in the other disease, atheroma.

And the hips?  Interestingly further tests showed the hips as having early “avascular necrosis”, too subtle to be picked up on x-ray, but clearly shown on MRI.  Now avascular necrosis “AVN” is a condition which results from impaired blood supply to the head of the femur and a number of cases have been seen in Hughes Syndrome.

I believe it is just conceivable that in Mrs P.A.’s case the condition was early enough for the symptoms to improve with better blood supply.  Here’s hoping a bright young rheumatology of orthopaedic research fellow will follow this lead up!

Professor Graham R V Hughes MD FRCP